Pulmonary metastases in children: results of surgical treatment.

A retrospective analysis, from 1965 to 1987, of 22 patients less than 18 years of age undergoing thoracotomy for pulmonary metastases from previously diagnosed malignancy, was performed. There were 15 males and seven females whose ages ranged from 8 months to 17, years. Ten patients had primary osteogenic sarcoma, five had Wilms' tumor, and seven had miscellaneous other tumors. A total of 41 thoracotomies were performed with no mortality. The overall survival rate was 54.5%, with an average survival of 6.2 years after initial diagnosis. The osteosarcoma group had a 50% survival rate after an average of 62 months from initial diagnosis, while the Wilms' tumor group had an 80% survival rate with a 100-month average. The remaining seven patients had a 29% average survival rate 62 months after diagnosis. Of the 12 patients undergoing wedge resections, two died upon follow-up 20 and 21 months after initial diagnosis. As opposed to the survivors in this group, both required more than four wedge resections upon initial thoracotomy. Two patients requiring extended resections, one for Ewing's sarcoma and one for hepatoblastoma, died 35 and 3 months after diagnosis, respectively. Of the eight patients undergoing lobectomy and/or segmentectomy, 75% died an average of 31.3 months after diagnosis. Ten patients had two or more thoracotomies for an average of 2.9, with a 40% survival rate. Of the 27% who presented with initial bilateral lung metastases, 33% survived. Forty-five percent of patients had a tumor-free interval of less than 12 months prior to thoracotomy, resulting in a 60% mortality rate.(ABSTRACT TRUNCATED AT 250 WORDS)

Pulmonary sequestration.

A review of 41 patients over a 15-year period with a diagnosis of pulmonary sequestration was undertaken. The most common presenting complaint was repeated infections of the sequestrated segment. Two of our patients were symptomatic since the neonatal period. On an average, each patient was admitted three times to the hospital before undergoing surgery. All our patients had abnormal chest x-rays. Two patients had severe purulent infection needing emergency resection of the sequestrated lobe. In our experience, almost all cases of pulmonary sequestration require surgery in the long-term.

Bronchogenic cysts.

Between 1967 and 1987, 26 patients with bronchogenic cysts were treated in our institution: 15 females and 11 males, ranging in age from 2 months to 14 years (average 6 years, 4 months). Only four patients (15%) were 1 year old or less. Seven patients presented with asymptomatic lesions discovered on chest film. Nineteen patients presented with respiratory symptoms. Of note are the four patients aged 1 year or less, who presented with dyspnea and cyanotic spells. Only one of them presented with dysphagia. All patients underwent routine chest films and tomography and, later, computed tomography (CT) scan of the thorax once the study became available. Barium esophagogram and bronchoscopy were performed as required. Contrary to what has been published concerning the intraparenchymal location of bronchogenic cysts, the majority of the cysts (65%) were located in the mediastinum, usually in close proximity to the carina, mainstem bronchi, trachea, esophagus, or pericardium. Only 27% (7 of 26) were intraparenchymal, and 8% (2 of 26) were situated in the inferior pulmonary ligament. A correct preoperative diagnosis was made in 19 patients (73%). Intrapulmonary lesions were removed by segmentectomy or lobectomy. Cysts located elsewhere were simply excised. Among the cysts adhering to the trachea, 50% (two of four) required suture of the membranous trachea after excision. Postoperative complications included one pneumonia, which responded to antibiotics, and one transient paresis of the right phrenic nerve. Follow-up averaged 17 months (range, 0 to 17 years). No sequelae were noted and all patients were cured.

Childhood primary pulmonary neoplasms.

Nine children (6 boys, 3 girls) were diagnosed with a primary endobronchial or pulmonary parenchymal neoplasm. The average age at diagnosis was 9 years. Presenting complaints included cough (7), fever (5), pulmonary infection (3), respiratory distress (3), weight loss (2), pain (2), and hemoptysis (1). Pulmonary x-rays showed persistent atelectasis, pneumonic infiltrates or mass lesions. A computed tomography scan was performed in 8. Five of six endobronchial tumors were diagnosed with bronchoscopy and biopsy. Treatment consisted of thoracotomy and pulmonary resection in 7 cases and laser resection in 2. The pathologic diagnoses were bronchial carcinoid (3), bronchial mucoepidermoid carcinoma (1), inflammatory pseudotumor (plasma cell granuloma) of the bronchus (2) and of the lung parenchyma (1), fibrosarcoma (1), and rhabdomyosarcoma (1). Postoperative chemotherapy was given only to the patient with pulmonary rhabdomyosarcoma; this child died. One child has developed a local recurrence while 7 children are alive and free of disease at an average of 2.4 years postresection. Pulmonary neoplasms are unusual in the pediatric age group and represent a wide spectrum of pathology. Including the present series, 383 tumors have been described. Seventy-six percent were malignant. Early investigation and surgical intervention are essential in children with persistent pulmonary symptoms or x-ray abnormalities. In most cases, the prognosis is excellent with complete surgical resection; however, malignancies other than bronchial adenoma are associated with significantly mortality.

Malignant small-cell tumor of the thoracopulmonary region: 'Askin tumor'.

Malignant small-cell tumor of the thoracopulmonary region (MSCT) is an uncommon neoplasm in children. We describe five cases diagnosed since 1981 that fulfill the criteria put forth by Askin et al. Surgery was performed for diagnosis or therapy in all patients. Two patients underwent open lung biopsy, only because of tumor extent. The other three had chest wall resections. All patients received radiotherapy and chemotherapy. Three patients presenting initially with extensive disease died at intervals of 2.5 to 7 months after diagnosis. Two patients are alive and disease-free at 16 and 24 months postdiagnosis. All five cases were reviewed for standard histology and differential immunohistochemistry. Electronmicroscopy and tissue cultures were done in 3 of the 5 patients. All five patients were neuron-specific enolase-positive. MSCT is an exclusion diagnosis not always readily made. Clinical course and pathologic expertise may point to the correct diagnosis.

Neurogenic tumors and VIP-induced diarrhea.

The authors report a case of cervical ganglioneuroblastoma associated with intractable watery diarrhea and hypokalemia. The probable physiopathologic mechanism of the diarrhea and its relation to the vasoactive intestinal peptide secretion (VIP) by the tumor are discussed.

Management of thyroid nodules in children: a 20-year experience.

The medical records and surgical slides of 58 patients with the diagnosis of thyroid nodules (solitary nodule in 50 patients) are reviewed. The most common cause of thyroid nodules in this series is follicular adenoma (27 patients or 46%). A nuclear scan (technetium or radioactive iodine) was performed in 55 patients, of which 40 showed a cold nodule. Twelve of the 40 cold nodules were malignant (30%). However, for solitary nodules the incidence of cancer is 27%. This last figure is significantly greater than the one recently reported by Hung et al (18.5%). Available diagnostic methods are reviewed and the clinical management as derived from our experience is presented.

Esophageal leiomyoma in children: two case reports and review of the literature.

Leiomyoma of the esophagus is not uncommon in the adult population but is rarely seen in children; only 20 cases have been reported in the pediatric population. In this paper we describe two cases of esophageal leiomyoma in female patients aged 6 and 13 years and review previous reports. Several differences were noted between the pediatric and adult population. The mean age in children is 14 years (range, 4 to 20 years). Leiomyoma appears 1.71 times more often in females than in males. Localized lesions are found in only 9%, whereas the diffuse form predominates in 91%. The entire esophagus may be involved 35% of the time, and encroachment on the cardia or upper stomach occurs in 70%. Leiomyomas associated with familial syndromes (familial leiomyoma and Alport's syndrome) occur in 22% of the cases. Major symptoms include dysphagia (86%), dyspnea (36%), vomiting (27%), retrosternal pain (27%), and coughing (22%). The initial diagnosis following contrast studies is most often achalasia. The diagnosis of leiomyoma is made only with subsequent endoscopy. Enucleation was performed in only 11% of the cases; surgical resection (including part of the stomach) was necessary in 78% with a 21% postoperative mortality. Esophageal leiomyoma should be considered in the differential diagnosis of mediastinal masses and esophageal obstruction. Accurate preoperative diagnosis is desirable in order to plan proper surgical treatment.

Congenital adenomatoid malformation of the lung: current management and prognosis.

A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.

The abdominoscrotal hydrocele.

While hydrocele is among the commonest inguinal anomalies in children, less than 20 cases have been reported of its extreme form, the abdominoscrotal hydrocele (ASH). This anomaly consists of a large inguinoscrotal hydrocele which communicates in an hour-glass fashion with a large "intra-abdominal" component. The latter lies deep to the narrow internal inguinal ring, but superficial to the peritoneal cavity proper, which is displaced superiorly and medially. The abdominal component of the ASH thus lies latero- and retroperitoneally, sometimes reaching the lower pole of the kidney. We report five ASH in four children under one year old. All four underwent complete resection of the ASH. If the abdominal portion of the hydrocele can not be delivered through a standard approach, we advocate a properitoneal approach as described for recurrent adult hernias. The external oblique, internal oblique and transversalis muscles are divided horizontally above the level of the internal inguinal ring. The peritoneal cavity is retracted superiorly, separating it from the ASH. By decompressing the scrotal component of the ASH, its abdominal part can be emptied through the narrow communication at the internal ring. In this fashion, the processus vaginalis can be identified and ligated deep to internal ring, and the floor of the inguinal canal is left intact. The pathophysiology of ASH is not clear. A one-way valve effect of the patent processus vaginalis may be one cause of the massive accumulation of peritoneal fluid in the ASH. Complete resection is curative, and the properitoneal approach should be considered.

The pathogenesis of intestinal atresia.

The records of 28 patients with duodenal and 31 with jejunoileal intestinal atresia or stenosis were studied. Vomiting and abdominal distention were the most prominent symptoms; an unusual colon may be present in jejunal as well as ileal obstruction and is not pathognomonic for ileal obstruction. Intestinal atresia is associated with a high incidence of preterm babies but with a low incidence of intrauterine growth retardation. Fifty-three infants were operated upon; the overall survival rate was 79 per cent. The high percentage of mortality in duodenal obstruction is due to associated malformations. Theories of the pathogenesis of intestinal atresia do not seem to fit current clinical and experimental evidence in all patients. Possibly, different factors play a role in separate situations.

[End to side porto-caval shunt in familial type II homozygote hypercholesterolemia (author's transl)]. / L'anastomose porto-cave termino-latérale dans l'hypercholestérolémie familiae de type II homozygote.

Two patients with homozygous type II familial hypercholemia have had significant improvement by end to side porta-caval shunt. Follow-up is respectively 1 and 4 years. When all the other treatment have failed in lowering blood cholesterol and L.D.L. levels, a portal diversion may help to prevent cardio-vascular atherosclerotic complications in the pediatric age.

The paediatric chest tube.

Chest tubes are placed to empty the pleural space of air or fluid which prohibits full lung expansion. The function of these tubes is dependent on adequate placement, effective drainage and frequent re-evaluation of the patient and the chest drainage system. Knowledge of the principles of chest tube drainage is important to evaluate adequately the function of a tube thoracostomy.

[Treatment of hyperthyroidism in childhood and adolescence (author's transl)]. / Traitement de l'hyperthyroïdie de l'enfant et de l'adolescent.

33 cases of hyperthyroidism have been treated at "L'Hôpital Sainte-Justine" of Montréal, during the period 1961-1974. Nearly all patients were submitted to medical treatment. 15 were cured with medical treatment only, and 18 had to be submitted to a subtotal thyroidectomy. These two groups are compared and show the clear advantage of surgery in the treatment of this disease. There was no major post-operative complication. Two patients became definitively hypothyroid. The mean follow-up is five years and six months.